TY - JOUR
T1 - Appendicealmucinous neoplasms
T2 - Diagnosis andmanagement
AU - Shaib, Walid L.
AU - Assi, Rita
AU - Shamseddine, Ali
AU - Alese, Olatunji B.
AU - Staley, Charles
AU - Memis, Bahar
AU - Adsay, Volkan
AU - Bekaii-Saab, Tonios
AU - El-Rayes, Bassel F.
N1 - Publisher Copyright:
© AlphaMed Press 2017.
PY - 2017/9
Y1 - 2017/9
N2 - Objective. Appendiceal mucinous neoplasms (AMNs) are a rare and heterogeneous disease for which clinical management is challenging. We aim to review the literature regarding modalities of treatment to guide the management of AMNs. Methods and Review Criteria.We conducted a PubMed search in February 2016 for English-language publications, using the terms “appendiceal, ” “appendix, ” “carcinoma, ” “cancer, ” “mucinous, ” “treatment, ” “genes, ” “target, ” “genomic, ” and terms listed in the articles’ subheadings. Published reports and abstracts from the American Society of Clinical Oncology meetings were also searched. Results. In this review, we summarize current data and controversies in AMN classification, clinical presentation, molecular alterations, treatment outcomes with regard to cytoreductive surgery, hyperthermic intraperitoneal chemotherapy (HIPEC), and the role of systemic chemotherapy. Conclusion. Appendiceal mucinous neoplasms are a heterogeneous group of tumors with a rising incidence. Treatment is based on stage and histology. Low-grade tumors are treated surgically with resection of the primary site in early stage disease, or peritoneal debulking and HIPEC in patients with advanced stage disease. Treatment of high-grade tumors requires further prospective trials, and options include debulking surgery and HIPEC with or without preoperative chemotherapy. Trials evaluating novel therapies based on the molecular profiling of AMN tumors are needed to evaluate therapeutic options in patients who are not surgical candidates.
AB - Objective. Appendiceal mucinous neoplasms (AMNs) are a rare and heterogeneous disease for which clinical management is challenging. We aim to review the literature regarding modalities of treatment to guide the management of AMNs. Methods and Review Criteria.We conducted a PubMed search in February 2016 for English-language publications, using the terms “appendiceal, ” “appendix, ” “carcinoma, ” “cancer, ” “mucinous, ” “treatment, ” “genes, ” “target, ” “genomic, ” and terms listed in the articles’ subheadings. Published reports and abstracts from the American Society of Clinical Oncology meetings were also searched. Results. In this review, we summarize current data and controversies in AMN classification, clinical presentation, molecular alterations, treatment outcomes with regard to cytoreductive surgery, hyperthermic intraperitoneal chemotherapy (HIPEC), and the role of systemic chemotherapy. Conclusion. Appendiceal mucinous neoplasms are a heterogeneous group of tumors with a rising incidence. Treatment is based on stage and histology. Low-grade tumors are treated surgically with resection of the primary site in early stage disease, or peritoneal debulking and HIPEC in patients with advanced stage disease. Treatment of high-grade tumors requires further prospective trials, and options include debulking surgery and HIPEC with or without preoperative chemotherapy. Trials evaluating novel therapies based on the molecular profiling of AMN tumors are needed to evaluate therapeutic options in patients who are not surgical candidates.
KW - Abdominal surgery
KW - Appendix carcinoma
KW - Review
UR - http://www.scopus.com/inward/record.url?scp=85029649368&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85029649368&partnerID=8YFLogxK
U2 - 10.1634/theoncologist.2017-0081
DO - 10.1634/theoncologist.2017-0081
M3 - Review article
C2 - 28663356
AN - SCOPUS:85029649368
SN - 1083-7159
VL - 22
SP - 1107
EP - 1116
JO - Oncologist
JF - Oncologist
IS - 9
ER -